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REVIEW OF CURRENT LITERATURE
Year : 2020  |  Volume : 21  |  Issue : 4  |  Page : 255-258

Hot topics in pediatric dermatology


Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Date of Submission19-Sep-2020
Date of Decision21-Sep-2020
Date of Acceptance22-Sep-2020
Date of Web Publication30-Sep-2020

Correspondence Address:
Dr. Dipankar De
Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Sector-12, Chandigarh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2319-7250.296850

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How to cite this article:
Bakshi S, De D. Hot topics in pediatric dermatology. Indian J Paediatr Dermatol 2020;21:255-8

How to cite this URL:
Bakshi S, De D. Hot topics in pediatric dermatology. Indian J Paediatr Dermatol [serial online] 2020 [cited 2020 Oct 27];21:255-8. Available from: https://www.ijpd.in/text.asp?2020/21/4/255/296850




  Hidradenitis Suppurativa in Pediatric Population Top


We discuss a study by Vaiopoulos et al.[1] describing the characteristics of hidradenitis suppurativa (HS) in the pediatric population at Dessau Medical Center, Germany. This was a retrospective study including 20 pediatric patients (1 child and 19 adolescents) who fulfilled the diagnostic criteria of HS. The median age at onset and diagnosis of HS was 15 and 17 years, respectively, with a male-to-female ratio of 1.86:1. The clinical site of involvement in the decreasing frequency included axilla (84%), groin (58%), submammary area (21%), buttocks (16%), and the pubic area (11%). A median body mass index of 25.2 reflected majority of the patients being either overweight or obese. Although most of the patients suffered from moderate disease severity, a significant proportion had associated comorbid disorders. Acne vulgaris/conglobate was the most frequent comorbid disorder seen in 68% of the patients followed by psoriasis (11%). A positive family history was reported in four out of 18 patients. Only seven patients (35%) were smokers. Further, a systematic review with 13 studies meeting the eligibility criteria revealed that most pediatric patients with HS were females, obese, and nonsmokers with an increased burden of comorbid disorders. However, the heterogeneity of the studies did not allow for a meta-analysis.


  Comments Top


This is one of the few studies discussing the demographics, clinical characteristics, and associated comorbidities in pediatric HS. The disease onset occurred mainly in the postpubertal age group corresponding to the attainment of adrenarche and rising hormonal influences. The majority of the patients in the study were males, which is in contrast to other studies on pediatric HS, which show a predominance of female patients.[2] The anatomical distribution of skin lesions observed in the study population was similar to the typical distribution of HS in adult patients. Obesity has been associated as risk factor in HS and may play a role in pathogenesis as well as disease exacerbation. A high incidence of obesity in pediatric HS patients was also reflected in this study. In a study by Balgobind et al.,[3] pediatric HS patients were 2.48 times more likely to be obese compared to those without HS. Another factor known to influence the course of HS is smoking, which in contrast to obesity was not so prevalent in the pediatric HS patients. This may be attributed to the restricted availability and legal implications of smoking in children. Furthermore, passive smoking which appears to be more relevant in pediatric patients could not be assessed in this study. Another retrospective analysis by Riis et al.[2] showed that 50% of pediatric patients were passive smokers. A positive family history has been associated with a younger age of onset and increased severity of disease in some studies.[4] No such association was demonstrated in this study cohort, and most of the patients had a moderate severity of the disease. A very high burden of comorbid disorders, especially acne, was present in patients. Psychological and endocrine disorders reported in some studies on pediatric HS were not observed in this study.

The drawbacks of this study were that it was a single-center study, resulting in a small sample size and its retrospective nature. This study highlights the need for screening for obesity and other associated comorbidities in cases of pediatric HS. Prospective studies on larger cohorts are needed to determine the outcome of childhood/adolescence-onset HS.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.


  References Top


  1. Vaiopoulos AG, Nikolakis G, Zouboulis CC. Hidradenitis suppurativa in paediatric patients: A retrospective monocentric study in Germany and review of the literature. J Eur Acad Dermatol Venereol 2020. https://doi.org/10.1111/jdv.16520.
  2. Riis PT, Saunte DM, Sigsgaard V, Villani AP, Guillem P, Pascual JC, et al. Clinical characteristics of pediatric hidradenitis suppurativa: A cross-sectional multicenter study of 140 patients. Arch Dermatol Res 2020. DOI: 10.1007/s00403-020-02053-6. PMID: 32166376.
  3. Balgobind A, Finelt N, Strunk A, Garg A. Association between obesity and hidradenitis suppurativa among children and adolescents: A population-based analysis in the United States. J Am Acad Dermatol 2020;82:502-4.
  4. Deckers IE, van der Zee HH, Boer J, Prens EP. Correlation of early-onset hidradenitis suppurativa with stronger genetic susceptibility and more widespread involvement. J Am Acad Dermatol 2015;72:485-8.



  Assessing Treatment Response of Pediatric Morphea to Steroids and Methotrexate; Where Does Multimodal Imaging Stand? Top


Here, we discuss a study by Weibel et al.[1] evaluating the treatment response and reversibility of skin lesions in response to steroids and methotrexate in 22 patients of pediatric morphea. The treatment protocol comprised of an initial induction phase of two courses of intravenous methylprednisolone (IVMP) (three pulses of 30 mg/kg/day [max 500 mg/day] on 2 consecutive weeks) in combination with oral prednisolone started after the first course of IVMP and then tapered gradually. This was followed by a maintenance regimen of weekly methotrexate (15 mg/m 2 body surface area, max 25 mg/week) which started 1 week after the second dose of IVMP for a minimum of 18 months up to a maximum of 8 years. Treatment response was assessed by using clinical scores as well as by determining the cutaneous blood flow, skin temperature and dermal thickness, and echogenicity by laser Doppler flowmetry (LDF), infrared thermography, and high-frequency ultrasound (HFU), respectively. The authors noticed a rapid cessation of the disease activity as well as improvement of the preexisting lesions in terms of resolution of dermal sclerosis. Imaging studies of the morphea lesions at baseline demonstrated an increase in blood flow with LDF and a diminished dermal thickness with increased echogenicity using HFU. Thermography did not show any significant differences in skin temperature. The findings of LDF and HFU but not thermography correlated with the disease activity in the active phase of the disease. However, follow-up studies using HFU did not show any significant trend, while LDF showed a steady decrease in blood flow with the treatment which did not correlate with the clinical scores. The relapse rates after stopping therapy were as high as 36% requiring reinstitution of the immunosuppressive therapy, with a median time to relapse being 20.3 months.


  Comments Top


Pediatric morphea is a challenging disorder to treat as the disease has to be arrested at an early stage to prevent disfigurement from permanent sequelae. Further, the treatment initiation and monitoring of disease activity are based on the clinician's assessment of the disease as various laboratory markers and imaging techniques have been suggested but not prospectively validated for the same. To our knowledge, this is the first study that combined the use of clinical scores with multiple imaging techniques to monitor disease activity as well as response to treatment in pediatric morphea. The efficacy of methotrexate as a monotherapy or as a maintenance therapy along with systemic steroids in the inflammatory stage of the disease has been established in the consensus treatment plans by Li et al.[2] A similar protocol was used to treat the patients in this study. According to the results of this study, LDF and HFU can be useful tools in guiding the initiation of therapy in morphea with their limited role in following up the patients during the treatment. The localized scleroderma cutaneous assessment tool which captures both activity and skin damage in morphea has been identified as a better outcome measure while determining the effectiveness of a therapy in pediatric morphea.[3] This has not been included in the current study due to its introduction at a later point of time. Another important finding that emerged was the reversibility of existing skin lesions noted as resolution of dermal sclerosis and even regrowth of hair in alopecia areas after starting treatment. The relapsing remitting course of morphea poses another challenge in the therapy as the exact treatment duration cannot be defined. This study also had high relapse rates with up to one-third of patients still being on therapy at the end of the 8-year follow-up period. In the study by O'Brien et al.,[4] 36% of patients had a disease relapse with a median time to disease recurrence being 1.1 years for generalized morphea and 2.3 years for linear subtype. Clinicians need to keep this in mind while counseling the patients and planning follow-ups after treatment discontinuation.

The strengths of this study include its prospective nature along with a long follow-up period of 8 years. However, the study included only a limited number of subjects, and due to the unavailability of a validated clinical score at the time of initiation of the study, a nonvalidated score was used. In conclusion, the use of multimodal imaging techniques can help guide the physicians about the need to initiation therapy, but the assessment of treatment response still relies on the clinical examination. In addition, the high cost, the limited availability, and the level of expertise and training required for performing these procedures make their routine use questionable.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.


  References Top


  1. Weibel L, Theiler M, Howell KJ, Denton CP, Waelchli R, Atherton D, et al. Prospective evaluation of treatment response and disease reversibility of paediatric localized scleroderma (morphoea) to steroids and methotrexate using multi-modal imaging. J Eur Acad Dermatol Venereol 2020;34:1609-16.
  2. Li SC, Torok KS, Pope E, Dedeoglu F, Hong S, Jacobe HT, et al. Development of consensus treatment plans for juvenile localized scleroderma: A roadmap toward comparative effectiveness studies in juvenile localized scleroderma. Arthritis Care Res (Hoboken) 2012;64:1175-85.
  3. Kelsey CE, Torok KS. The Localized Scleroderma Cutaneous Assessment Tool: Responsiveness to change in a pediatric clinical population. J Am Acad Dermatol 2013;69:214-20.
  4. O'Brien JC, Nymeyer H, Green A, Jacobe HT. Changes in disease activity and damage over time in patients with morphea. JAMA Dermatol 2020;156:513-20.



  Risk Factors Associated with Ulceration of Lip Infantile Hemangiomas Top


Next, we discuss a study by Yu et al.[1] that aimed to describe the clinical characteristics of lip infantile hemangiomas (IHs) as well as risk factors associated with their ulceration. In this retrospective study, 69 cases of lip IH that fulfilled the inclusion criteria were reviewed. Of note, previously treated lip IH and perioral hemangiomas not involving the vermilion of lip were excluded from the study. There was a slight female preponderance with female-to-male ratio being 1.4:1. Fifteen cases (21.7%) were premature infants and 14 (20.3%) had a history of low birth weight. The mean age at onset of hemangioma was 11.3 days. The IH involved the upper lip in 31 (44.9%) and lower lip in 38 (55.1%) patients. The distribution of IH on the right side, left side, and medial lip was 27 (39.1%), 21 (30.4%), and 5 (7.3%), respectively. Sixteen patients (23.2%) had involvement of the whole vermilion and 30 (43.5%) had extension of hemangioma to the oral mucosa or gingiva. Sixteen patients had segmental IH and another 15 patients had multifocal hemangiomas, with the most common site of involvement being cheek, jaw, nose, eyelid, and scalp. None of the patients had airway or liver hemangiomas. Ulceration developed in 37 (53.6%) patients. The mean size of IHs developing ulceration was 3.49 cm 2 as compared to 1.08 cm 2 for those that did not develop any ulceration. At the end of the study, the location of IH at the lower lip and the size of the IH were identified as risk factors predicting ulceration. Swelling of the IH that either turns pale or bright crimson could also point toward impending ulceration.


  Comments Top


IHs are the most common benign vascular tumors in children that usually follow a predictable course, with an initial proliferative phase followed by a maturation phase and finally involution phase. However, this benign course can be interrupted by several complications raising the need for active intervention. These include ulceration, hemorrhage, airway obstruction, and superinfection among others. Identification of risk factors that can predict these complications can timely alert the physicians for the need of aggressive management of these IHs and thus prevent any adverse outcomes. Lip IHs pose a greater risk as they can interfere with speech and feeding and are more susceptible to ulceration that may cause esthetically unacceptable disfigurement. The authors have hypothesized that sucking of the lips by infant, abrasions during speech and feeding, and rapid growth of the tumor causing ischemia, may all contribute to the increased incidence of ulceration of lip IHs. More than half of the patients developed ulceration in this study as compared to only 23% of the patients developing ulceration in the study by Herman et al.[2] This discrepancy might be explained by the inclusion of only untreated IHs located on the lip which are more prone to develop ulceration. Location of the IH on the lower lip and greater size were the risk factors associated with ulceration in this study. In the study by Yanes et al.,[3] IHs having a large size or located on the upper lip and vermilion border were associated with poor outcomes.

The strengths of this study include a relatively large sample size with factoring in of multiple variables as possible risk factors. The drawbacks include retrospective nature of the study with a possible recall bias. Further, the data were collected at a single center which makes the extrapolation of the study results to the general population debatable. Since prospective evaluation of risk factors for ulceration of lip IHs might not be feasible owing to ethical issues, data from multiple centers may be pooled to corroborate the findings of the study.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.


  References Top


  1. Yu L, Li LL, Yan P, Deng L, Gan XL, Yao XJ, et al. Clinical characteristics of lip infantile haemangiomas and main risk factors for ulceration: An 8-year retrospective study of 69 Chinese infants. J Eur Acad Dermatol Venereol 2020. PMID: 32118308. DOI: 10.1111/jdv.16331.
  2. Hermans DJ, Boezeman JB, Van de Kerkhof PC, Rieu PN, Van der Vleuten CJ. Differences between ulcerated and non-ulcerated hemangiomas, a retrospective study of 465 cases. Eur J Dermatol 2009;19:152-6.
  3. Yanes DA, Pearson GD, Witman PM. Infantile hemangiomas of the lip: Patterns, outcomes, and implications. Pediatr Dermatol 2016;33:511-7.



  Drug Reaction with Eosinophilia and Systemic Symptoms in Pediatric Population Top


Here, we discuss a systematic review by Metterle et al.[1] reviewing the literature on drug reaction with eosinophilia and systemic symptoms (DRESS) in the pediatric population. One hundred and nineteen studies comprising 130 probable or definite cases of DRESS were analyzed for demographic profile, causative drug with the latent period, clinical symptoms, and laboratory investigations, including viral reactivation, treatment received, and the outcomes. The average age at diagnosis was 8.7 years with no gender predilection. Seizure was the most common comorbidity. The culprit drugs included antiepileptics (50%), antibiotics (30.8%), and sulfasalazine and nonsteroidal anti-inflammatory drugs (4.6%). Carbamazepine, phenytoin, phenobarbital, and lamotrigine were the major antiepileptics, whereas vancomycin, trimethoprim-sulfamethoxazole, and amoxicillin ± clavulanate were the major antibiotics implicated. The symptom onset occurred at an average of 23.8 days after starting drug intake, with a mean of 25.5 and 19.2 days of intake for antiepileptics and antibiotics, respectively. The clinical symptoms included rash (99.2%), fever (96.2%), eosinophilia (90%), lymphadenopathy (74.6%), and edema (59.2%). HHV-6 testing was done in 37.7% of cases and reactivation occurred in 16.1% of cases, overall. The most commonly involved internal organ was the liver (80%), spleen (21.5%), and kidney (15.4%). The offending drug was discontinued followed by the initiation of systemic corticosteroids (88.5%), intravenous immunoglobulin (12.3%), plasmapheresis (2.3%), ganciclovir (1.5%), or a combination of these. Long-term sequelae were seen in 10.8% in the form of hypothyroidism (3.8%), liver failure (3.1%), and diabetes mellitus (2.3%). The mortality rate was 5.4% with the cause of death being attributed to DRESS in five cases and nosocomial infections in the other two.


  Comments Top


DRESS is a severe cutaneous adverse drug reaction that can have high mortality and morbidity if the suspected drug is not discontinued at an early stage. The diagnosis of DRESS can be made by keeping a high index of clinical suspicion and eliciting a temporal correlation between the drug intake and onset of rash. It can further be guided by various scoring guidelines and appropriate laboratory investigations. Making a diagnosis of DRESS in the pediatric age group can be all the more challenging, due to various mimics such as viral infections, Kawasaki disease, and pseudolymphoma. This study defined the characteristics of pediatric DRESS in detail. Antiepileptics, particularly aromatic antiepileptics, and antibiotics were the most commonly incriminated drugs in this study, similar to the results of the systematic review of pediatric DRESS by Kim et al.[2] Carbamazepine is also the most common drug associated with DRESS in adults.[3] Skin involvement is almost a universal finding in pediatric DRESS, with eosinophilia and lymphadenopathy being commonly observed. Facial edema is a relatively less frequent finding. Particular importance needs to be given to liver abnormalities as it is the most frequently involved internal organ. Palpation of the spleen during physical examination should be emphasized as spleen is the next most commonly involved organ. Furthermore, long-term follow-up is required in all cases because of sequelae in the form of endocrinological abnormalities. The management of patients relies mainly on removal of the offending agent and systemic corticosteroids. The mortality rates in pediatric and adult DRESS are comparable.

To conclude, clinicians prescribing certain drugs notorious for causing DRESS should closely monitor the patients and stop the drug at the earliest sign of a drug rash.

The limitations of this study include its retrospective nature with variability in data documentation and inclusion of probable DRESS cases along with the definite cases.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.


  References Top


  1. Metterle L, Hatch L, Seminario-Vidal L. Pediatric drug reaction with eosinophilia and systemic symptoms: A systematic review of the literature. Pediatr Dermatol 2020;37:124-9.
  2. Kim GY, Anderson KR, Davis DMR, Hand JL, Tollefson MM. Drug reaction with eosinophilia and systemic symptoms (DRESS) in the pediatric population: A systematic review of the literature. J Am Acad Dermatol 2020. PMID: 32247873. DOI: 10.1016/j.jaad.2020.03.081.
  3. Cacoub P, Musette P, Descamps V, Meyer O, Speirs C, Finzi L, et al. The DRESS syndrome: A literature review. Am J Med 2011;124:588-97.





 

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